related links

search string

by alphabet

by localization

Progressive Systemic Scleroderma

definition

A chronic, progressive dermatosis characterized by boardlike hardening and immobility of the affected skin, with visceral involvement, especially of lungs, esophagus, kidneys and heart. It may be accompanied by calcinosis, Raynaud's phenomenon, and telangiectasis (CREST syndrome). It includes acrosclerosis and sclerodactyly. The pattern of scleroderma occuring in childhood differs from that in the adult. Childhood onset of progressive scleroderma is rare.